Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical and pathological findings are reported in a Japanese girl who died of secondary amyloidosis associated with juvenile rheumatoid arthritis two years after the onset of symptoms. The patient had intermittent high fever, rheumatoid rash, polyarthralgia, and hepatosplenomegaly. The joints showed the typical histologic changes of juvenile rheumatoid arthritis. Amyloid deposition was found in various tissues; however, remarkable deposition of amyloid was observed in the gastrointestinal tract, especially in the ileum. The amyloid protein in this patient was identified as protein AA using the methods of potassium permanganate treatment and the peroxidase-antiperoxidase unlabeled antibody technique.
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PMID:Amyloidosis associated with juvenile rheumatoid arthritis. 707 94

A 23-yr-old woman in her sixth month of pregnancy presented with the systemic variant of juvenile rheumatoid arthritis (Still's disease). The symptoms included high fever, erythematous evanescent maculopapular rash, hepatosplenomegaly and asymmetric arthritis. During a follow-up period of 1 yr, anti-inflammatory drugs (both steroidal and nonsteroidal) were administered, leading to the eventual disappearance of the patient's complaints. Adult-onset Still's disease reported in the literature is reviewed, and the association of Still's disease with pregnancy is discussed.
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PMID:Adult Still's disease associated with pregnancy. 717 71

The clinical features of 13 patients with angioimmunoblastic lymphadenopathy were analyzed to determine prognostic factors and response to therapy. Eleven patients presented with sudden onset of fever, weight loss, generalized lymphadenopathy, and hepatosplenomegaly. Laboratory features included autoimmune hemolytic anemia and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in six cases and skin rash in four. Two patients had localized lymphadenopathy without systemic symptoms. Both are alive at 5.5 and 2.5 years, respectively, after diagnosis, although the latter patient has required intermittent prednisone for recurrent lymphadenopathy. An additional patient is alive on treatment for months following diagnosis. The remaining ten have died, nine of sepsis and one of cerebral hemorrhage. The immunosuppression and myelosuppression of combination chemotherapy may have hastened their deaths. An individualized, conservative treatment approach is recommended.
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PMID:Angioimmunoblastic lymphadenopathy: clinical spectrum of disease. 729 97

Immunological studies were carried out on two female patients with angioimmunoblastic lymphadenopathy (AIL). Both presented with fever, lymphadenopathy, hepatosplenomegaly, rash and apparent ampicillin hypersensitivity. During the active phase of the disease, cellular immunity was depressed and T cell blastogenesis induced by lectins was abnormal. In the first patient, a non-dialysable plasma factor was found that inhibited normal lymphocyte blastogenesis, the removal of which enhanced the activation of AIL lymphocytes. This inhibitory plasma factor was also observed in the second patient during relapse of the disease. The latter patient responded well to steroid and levamisole therapy, showing clinical remission and a return of in vivo and in vitro parameters of cellular immunity. Defective B cell regulation due to impaired suppressor function, followed by immunoglobulin overproduction, is suggested to occur in AIL.
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PMID:Immunological studies in angioimmunoblastic lymphadenopathy. 738 5

Diagnosis of Hodgkin's disease was made in a patient who had been treated with diphenylhydantoin sodium for 4 years. Three different neoplastic diseases of the lymphoid tissue following therapy with hydantoin derivatives have been reported. Pseudolymphoma is characterized by lymph node enlargement, fever, arthralgias, cutaneous rash, hepatosplenomegaly and eosinophilia. The lymph node biopsy reveals a non-malignant histopathologic pattern. Pseudolymphoma develops few weeks after administration of hydantoin and it completely disappears when medication is discontinued. Pseudo-pseudolymphoma has similar clinical and histologic characteristics than those of pseudolymphoma, but patients in this category develop a true lymphoma after an asymptomatic period. Lymphomas (Hodgkin's disease and non-Hodgkin's lymphomas) appear following prolonged treatments with hidantoin derivatives. Risk to develop lymphoma is two to four times higher for patients taking such medication. Carcinogenic mechanism of hydantoin is unknown, but it might be related to the immunosuppressive effect of this drug.
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PMID:[Hodgkin's disease following treatment with hydantoins. Report of a case and review of the literature (author's transl)]. 739 7

Angioimmunoblastic lymphadenopathy is a rare clinical entity, first described in 1974, characterized by asthenia, anorexia, fever, sweating, generalized lymph node enlargement, hepatosplenomegaly, rash, hypergammaglobulinemia, and often Coomb's positive hemolytic anemia. Main histopathologic findings are lymphoplasmocytic and immunoblastic proliferations, increased vascular neshwork and interstitial granular PAS positive material deposits. A lymph node excised from a woman with angioimmunoblastic lymphadenopathy was examined under electron microscopy. Results of ultrastructural study are compared to the typical histologic pattern observed under light microscopy. Analysis of the cellularity and the significance of fibrous collagen found in the interstitial PAS positive material are commented on.
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PMID:[Angioimmunoblastic lymphadenopathy. Case report with ultrastructural study (author's transl)]. 741 34

CD56, a natural killer cell marker reactive with the neuronal-cell adhesion molecule (NCAM), identifies a group of lymphomas with distinctive clinicopathologic features. The disease affects mostly middle-aged adults who often present with fever, skin rash and hepatosplenomegaly in the absence of peripheral lymphadenopathy. Extranodal involvement is common, particularly the skin, aerodigestive tract and central nervous system. Histologically, an angiocentric and angiodestructive pattern of infiltrate is often seen, but the cytological spectrum of the lymphoma cells is very broad. Cytoplasmic granules, however, are frequently found when Giemsa-stained cytologic preparations are examined. Immunologically, CD56-positive lymphomas can be sub-classified into CD3-positive (T-cell) and CD3-negative (probably true natural killer cell) subtypes. T-cell receptor gene rearrangement can be demonstrated in the former cases, but not in the latter. Clinically, CD56-positive lymphomas are aggressive neoplasms.
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PMID:CD56 (NCAM)-positive malignant lymphoma. 752 19

Langerhans cell histiocytosis (LCH) is a class I histiocytosis characterized by the presence of the pathologic Langerhans cell, an unique histiocyte. In contrast to LCH, class II histiocytosis is characterized by the proliferation of mononuclear phagocytes other than Langerhans cells and includes sinus histiocytosis with massive lymphadenopathy, viral-associated hemophagocytic syndrome, and familial hemophagocytic lymphohistiocytosis. Until now, these two classes have been considered separate, if related, entities. We report a 10-month-old girl who presented with pyrexia, hepatosplenomegaly, an eczematous skin rash, anemia, thrombocytopenia, and a markedly elevated serum IgG and IgM antibody level to cytomegalovirus. Histologic proof of both hemophagocytosis in the liver and bone marrow and LCH in the skin was obtained at presentation. The clinical course and response to treatment over 6.5 years is recorded. Although the etiology of both class I and class II histiocytosis remains unknown, we speculate that the monocytic/macrophage disorder, as well as the LCH, were both triggered by virus or viral-related monokines secreted by activated macrophages.
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PMID:Simultaneous occurrence of viral-associated hemophagocytic syndrome and Langerhans cell histiocytosis: a case report. 762 81

We present two pseudolymphoma occurring 8 days and 1 month after carbamazepine introduction. Both patients present fever, rash, generalized lymphadenopathy and hepatosplenomegaly in the second case. Hematologic abnormalities included anemia, eosinophilia, hepatic cytolysis. Histologic evaluation of a lymph node biopsy specimen demonstrated near-total effacement of the nodal architecture mimicking a lymphoma. Gene rearrangement studies proved the benign nature of the proliferation. Carbamazepine-induced lymphoproliferative disorders are relatively rare with only 38 observations published. The pathogenesis is uncertain. Immune dysregulation is probable. Morphologic and immunophenotypic data must be completed by gene rearrangement studies. Corticoid therapy is useless. The evolution is favorable after the cessation of carbamazepine.
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PMID:[Pseudolymphoma induced by carbamazepine. Apropos of 2 cases]. 774 Feb 34

We present the case of a 67-years-old patient which, after treatment with ciprofloxacin, developed fever, exanthema, arthralgias, polyadenopathies, hepatosplenomegaly, autoimmune hemolytic anemia, hypergammaglobulinemia and severe inversion of the CD4/CD8 ratio. Latter, he developed ischemic signs in several locations (splenic and cerebral infarcts), as well as polyneuropathy and inflammatory myopathy. The diagnosis of angioimmunoblastic lymphadenopathy was established through ganglionary biopsy. The patient improved initially with the administration of corticoids, but in a few months, he developed pleomorphic T lymphoma with quick fatal evolution. We discuss the rarity of some of the clinical and laboratory manifestations in this patient and we comment on the association of ciprofloxacin and the angioimmunoblastic lymphadenopathy, which has never been previously described in the international literature.
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PMID:[Angioimmunoblastic lymphadenopathy. Apropos a case with unusual clinical manifestations and evolution to T-cell lymphoma]. 785 88


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